PRECIPITATING ANTIBODIES TO FACTOR-VIII-VONWILLEBRAND FACTOR IN VONWILLEBRANDS DISEASE - EFFECTS ON REPLACEMENT THERAPY

  • 1 January 1981
    • journal article
    • research article
    • Vol. 57  (1) , 25-31
Abstract
Precipitating antibodies to factor VIII/von Willebrand factor can develop in patients with severe homozygous-like von Willebrand''s disease following multiple transfusions with blood derivatives. This study of 4 patients treated with cryoprecipitate for 13 different bleeding episodes demonstrates that the occurrence of such antibodies interferes with the disease management. The control of mucosal bleeding was poor, whereas more favorable responses were obtained in soft-tissue hemorrhages. These findings probably relate to failure of replacment therapy to shorten the prolonged bleeding time. Immediately after treatment, measurement of plasma factor VIII/von Willebrand factor-related antigen and ristocetin cofactor showed either no increase, or very low values, depending on the pre-infusion antibody titer. Factor VIII/von Willebrand factor-related procoagulant activity levels in the circulation were also lower than predicted and usually there was no evidence of the delayed and sustained rise typically observed in uncomplicated von Willebrand''s disease. An anamnestic rise in antibody titer appeared 6-15 days after treatment and showed no obvious relationship with the cryoprecipitate amount infused. Replacement therapy invariably caused severe side effects during, or immediately after, concentrate infusion. In vitro studies, results support the view that these reactions were due to the appearance of circulating immune complexes.